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PubMed1996

Myocarditis--cardiomyopathy. Consensus Report of the German Association for Internal Medicine, presented at the 100th annual meeting, Wiesbaden, 13 April 1994.

On the basis of several, partially divergent definitions, myocarditis-unlike the often aetiologically unexplained (idiopathic) cardiomyopathies (dilated, hypertrophic, restrictive)-can be classified as c specific disease of the myocardium. This disease often has a known cause and can be due to a large variety of aetiological agents (viral, autoreactive, toxic, infectious processes, etc.) (1). A question still to be answered is whether there is a connection between dilated cardiomyopathy and myocarditis, i.e. whether (virus-induced) myocarditis can progress via a subacute or chronic stage to dilated cardiomyopathy. This question is of considerable diagnostic and therapeutic relevance. The following consensus report thus deals with the clinically important problems related to the diagnosis of myocarditis and dilated cardiomyopathy, examines the aetiological role of immunological mechanisms and discusses the treatment of the two diseases and prognostic factors.

Cardiomyopathy, Dilated↗
PubMed2006

Alterations in cardiac connexin expression in cardiomyopathies.

Gap junctions, assembled from connexins, form the cell-to-cell pathways for propagation of the precisely orchestrated patterns of current flow that govern the synchronized rhythm of the healthy heart. As in most tissues and organs, multiple connexin types are co-expressed in the heart; the connexins Cx43, Cx40 and Cx45 are found in distinctive combinations and relative quantities in different, functionally specialized subsets of cardiomyocytes. Alterations in connexin expression and gap junction organization, now a well-documented feature of human cardiomyopathies, potentially contribute to the pro-arrhythmic substrate. In the diseased ventricle, the most consistently reported quantitative alteration involves heterogeneous reduction in Cx43 expression and disruption of the normal ordered pattern of Cx43 gap junction distribution. Additional studies suggest that upregulation of Cx40 and Cx45 may also feature in the failing ventricle, the former restricted to ischemic cardiomyopathy and localized to the subendocardial region. By correlating data from studies on the human patient with those from animal and cell models, alterations in connexin expression and gap junction organization have emerged as important factors to be considered in understanding the pro-arrhythmic substrate found in human cardiomyopathies.

Animals↗
PubMed2003

First-degree atrioventricular block and restrictive physiology as cardiac manifestations of Fabry's disease.

Fabry's disease is an X-linked disorder of glycosphingolipid catabolism related to the defective activity of glycosphingolipid, mainly ceramide trihexoside, in the vascular smooth muscle, myocardium, cells of the sympathetic central nervous system, and epithelial cells of renal glomeruli. We describe a young man who had Fabry's disease and unusual electrocardiographic and echocardiographic patterns at admission for treatment of left leg cellulitis. Findings included a prolonged PR interval and a right bundle branch block pattern, no echocardiographic signs of septal or hypertrophic cardiomyopathy, and a restrictive physiologic pattern. This pattern of electrocardiographic and echocardiographic characteristics of Fabry's disease has not been reported previously and should be added to the other cardiac manifestations of Fabry's disease.

Adult↗
PubMed2000

Health related quality of life and psychological wellbeing in patients with dilated cardiomyopathy.

OBJECTIVE: To assess the health related quality of life and psychological wellbeing of patients with dilated cardiomyopathy, and relate these to clinical variables and psychological adjustment. DESIGN: Postal questionnaire survey of 99 adult patients with dilated cardiomyopathy, selected at random from a larger database (60.6% response rate). Assessments included the short form 36 (SF-36) health survey, the hospital anxiety and depression scales, the sleep problems index, and a measure of psychological adjustment to cardiomyopathy. RESULTS: Patients with dilated cardiomyopathy reported significant impairments in physical functioning, role limitations owing to physical and emotional problems, social functioning, mental health, perceptions of general health, sleep, and vitality. Anxiety and depression levels were higher than in population samples. Impairment in several domains of quality of life was associated with low shortening fraction, high left ventricular end diastolic diameter, and the presence of heart failure and mitral regurgitation. Patients with familial cardiomyopathy had less impairment in quality of life than those with non-familial disease. Psychological adjustment scores were associated with several aspects of quality of life and emotional wellbeing. In multivariate analysis, demographic and clinical variables accounted for 0.1-40.7% of the variance in different domains of quality of life, and psychological adjustment scores accounted for an additional 0.5-22.4% of variance. CONCLUSIONS: Patients with dilated cardiomyopathy experience pronounced restrictions in quality of life and psychological wellbeing. These limitations are only partly accounted for by symptoms and the severity of underlying disease. Patients may benefit from efforts to improve psychological adjustment to the condition.

Adolescent↗
PubMed2006

A hemizygous SCO2 mutation in an early onset rapidly progressive, fatal cardiomyopathy.

Mutations in SCO2, a metallochaperone involved in mitochondrial copper delivery, are associated with early onset, fatal hypertrophic cardiomyopathy. All reported patients carry at least one copy of the common 1541G>A (E140K) mutation. Whereas patients with one copy of the E140K allele, in combination with a more deleterious mutation, follow a severe clinical course, patients homozygous for the E140K mutation have a delayed onset of disease and a more prolonged survival. Here, we have investigated a patient who appeared homozygous for the common 1541G>A mutation based on DNA sequencing and restriction enzyme analysis of a PCR product, yet presented with early onset, severe cardiomyopathy. Restriction enzyme analysis of parental DNA revealed that the mother was heterozygous for 1541G>A, while the father was homozygous wild-type. The patient showed biparental inheritance for microsatellite markers spanning the length of chromosome 22, making isodisomy unlikely. Sequencing of several single nucleotide polymorphisms within the 5'-UTR, intron and single exon of the SCO2 gene was uninformative; however, a 16 bp deletion within the intron was present in the patient and the mother, but not the father. Restriction enzyme analysis confirmed that the mother was heterozygous and that the patient was hemizygous for the deletion. Southern blot, Northern blot, and FISH analyses were consistent with the de novo deletion of one allele of SCO2 in the patient. This is the first report of hemizygosity in a SCO2 patient. The patient phenotype underscores the strikingly similar clinical course in all patients with one copy of the E140K allele. Examination of both patient and parental genotypes by thorough molecular analyses can reveal information with important implications for genetic counseling.

Cardiomyopathies↗
PubMed2001

Clinical aspects of left ventricular diastolic function assessed by Doppler echocardiography following acute myocardial infarction.

LV systolic function and dilation after Ml have been extensively studied and have been related to heart failure and cardiac mortality. In recent years, it has been increasingly apparent that LV diastolic dysfunction contributes to signs and symptoms of heart failure and LV diastolic dysfunction is associated with increased mortality rates in patients chronic heart failure independent of systolic function. LV diastolic dysfunction is difficult to assess on basis of clinical examination including chest radiography and electrocardiography. LV diastolic filling has traditionally been evaluated by cardiac catherization with direct measurement of filling pressures and relaxation. However, the invasive approach describing LV compliance and relaxation as the major determinants of LV diastolic function, is not feasible and suitable for routine investigations of diastolic function. Two-dimensional and Doppler echocardiography has become a well accented practical and safe non-invasive method for diagnosis of LV diastolic dysfunction. Combined invasive and echocardiographic studies have shown that analysis of mitral and pulmonary venous flow velocities relate to invasively measured filling pressures and relaxation rate in cardiac diseases. Based on Doppler analysis of mitral and pulmonary venous flow three abnormal LV filling patterns are identified: impaired relaxation, "pseudonormalization" and restrictive. These LV filling patterns have been related to symptoms, relaxation rate, filling pressure and prognosis in patients with restrictive and dilated cardiomyopathy. The Doppler flow profiles are influenced by several factors including age, heart rate, load conditions and valve heart diseases which must be taken into consideration during evaluation. During the last decade information about LV diastolic function assessed non-invasively by Doppler echocardiography has gained in patients with CAD. Myocardial ischemia induced by brief coronary artery occlusion or pacing leads to abnormal myocardial relaxation which can be reversed to normal by restoring normal myocardial blood flow. The diastolic abnormality is present within seconds and a characteristic impaired relaxation filling pattern are identified by mitral and pulmonary venous flow analysis. Diastolic dysfunction has been recognized during the early as well during the post-MI phase with or without LV systolic dysfunction. In the acute phase both an abnormal relaxation pattern and restrictive LV filling pattern are present which has been related to in-hospital heart failure. The identification of a pseudonormal or restrictive LV filling pattern are associated with later readmission to hospital with heart failure and cardiac death. Abnormal relaxation filling is the most pronounced filling pattern after one year which might be related to the remodeling process including compensatory hypertrophy, scarring of the infarct zone leading to a non-uniform relaxation of the LV. Remodeling of the LV following a MI is subject to several factors which might involve diastolic function. This is supported by the presence of an impaired relaxation and restrictive filling pattern are associated with progressive LV dilatation following Ml. Furthermore, the LV remodeling process following the very early phase includes the scarring process with collagen deposition in the infarcted and non-infarcted myocardium. The extent and quality of the repair process involving collagen deposition are believed to influence the remodeling process. Increased collagen deposition in the subacute phase of Ml indicated by elevated values of the collagen marker PIIINP is found to be related to LV dilation, depressed systolic function and restrictive LV filling. Development of a restrictive filling in patients with increased collagen deposition might be due to increasing LV volume but also to increased myocardial stiffness. Regarding prognosis diastolic dysfunction seems to be an important marker of outcome as abnormal diastolic properties are related to progressive LV dilatation, development of heart failure and cardiac death following MI. The beneficial effects of BB on morbidity and mortality in post-MI patients are well established. Recently, it has been demonstrated that BB has beneficial effects on progressive CHF and cardiac mortality in patients with chronic heart failure and moderate to severe systolic dysfunction. The mechanisms behind these effects are not fully understood. However, improvement of both systolic and diastolic function during BB therapy are demonstrated in patients with CHF. A few studies in patients with MI indicates that long-term BB therapy improves LV diastolic function which seems to be followed by improvement in systolic function. BB has the potential to lengthening diastole, improving subendocardial myocardial perfusion and affecting symptomatic amd neurohumoral activation following MI which might affect LV systolic and diastolic function and thereby improving outcome. Functional capacity following Ml is a well known predictor for outcome in MI patients. LV diastolic function a closely related to exercise capacity in contrast to measures of systolic function. BB therapy in patients with mild to moderate systolic dysfunction seems to improve exercise capacity which is related to improvement in LV diastolic function. Thus, BB improves exercise capacity and diastolic function by increasing LV compliance which might have prognostic implications. Even though LV systolic and diastolic dysfunction coexist, few two-dimensional and Doppler echocardiographic variables combine measurements of both phases of the cardiac cycle. Recently, the MPI has been suggested as a measure of combined systolic and diastolic myocardial performance which is based on Doppler time intervals of the systolic and diastolic phases. The MPI is easily obtained, reproducible, non-geometric and seems less dependent on heart rate and load conditions compared to traditional Doppler measurements. In patients with MI is has shown to reflect disease severity and contain prognostic information. The assessment of MPI seems therefore to be a relevant attractive alternative to established measurements of LV function following MI.

Diastole↗
PubMed1995

The arrhythmogenic right ventricle. Dysplasia versus cardiomyopathy.

Twenty-four patients presenting with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ventricular tachycardia of right ventricular origin associated with structural abnormalities of the right ventricle) were divided into two groups with left ventricular ejection fraction (LVEF) above or below 45%. The distribution of LVEF in the group with LVEF below 45% was comparable with the distribution in 6 patients with idiopathic dilated cardiomyopathy who had ventricular tachycardia originating in the left ventricle (P = 0.2). They also had the same unfavorable long-term prognosis. Therefore, it is suggested that the term, arrhythmogenic right ventricular cardiomyopathy (ARVC), be restricted to patients with a LVEF below 45%. Histological data obtained in the ARVC group showed signs of acute or chronic myocarditis (in the right and left ventricles). It can be hypothesized that patients with arrhythmogenic right ventricular dysplasia (ARVD) may be prone to develop infectious myocarditis. In patients in whom an abnormal host immune response had been seen, progressive deterioration of right and left ventricular function could be observed. This pattern may be superimposed on the genetically determined background of ARVD. This could explain the wide spectrum of clinical presentation observed in patients with tachycardia originating in an abnormal right ventricle.

Adolescent↗
PubMed1997

Doppler evaluation of left and right ventricular diastolic function: a technical guide for obtaining optimal flow velocity recordings.

Doppler mitral flow velocities and related variables are used to assess left (LV) and right ventricular filling and, indirectly, ventricular diastolic function. Three abnormal ventricular filling patterns (impaired relaxation and pseudonormal and restrictive physiology) are recognized in patients with various heart diseases and have been related to alterations in LV diastolic properties and filling pressures. More recently, these variables have been used to assess the hemodynamic effects of drug therapy or heart surgery and prognosis in patients with restrictive and dilated cardiomyopathies. Despite these encouraging results, widespread clinical use of these Doppler techniques has been hampered by difficulties in obtaining accurate and reproducible measurements from Doppler flow velocity recordings. This is due, in part, to an underappreciation of factors such as cardiac filling mechanics, Doppler examination principles, and ultrasound machine settings, which can markedly affect the quality of the flow velocity recordings. The purpose of this article is to provide the technical information for performing a systematic and comprehensive Doppler evaluation of LV diastolic function that can be used on a routine basis. This information includes discussing the different flow velocity recordings required for a Doppler assessment of LV diastolic function, their proper recording technique, and the common technical pitfalls.

Blood Flow Velocity↗
PubMed2002

Strain and strain rate echocardiography.

Strain and strain rate echocardiography is an emerging technique for assessing myocardial systolic and diastolic function. It is envisioned that this modality could change the quantitative assessment of regional wall motion and improve the accuracy and reproducibility of test readings. Myocardial strain and strain rate can detect inducible ischemia and at earlier stages than visual estimation of wall motion or wall thickening parameters. Changes in systolic strain rate and strain have potential to discriminate between different myocardial viability states. Measurement of diastolic rate of deformation can differentiate physiologic from pathologic hypertrophy, and restrictive from constrictive cardiomyopathy. This article reviews basic principles and current experimental and clinical applications of strain and strain rate echocardiography.

Animals↗
PubMed1993

Pitfalls in the echo-Doppler assessment of diastolic dysfunction.

The Doppler echocardiographic assessment of diastolic function is an essential part of the evaluation of heart failure, pericardial diseases, restrictive and infiltrative cardiomyopathies, and many other conditions. However, the echocardiographic evaluation of diastolic function has several limitations. The sonographer and physician must understand the technical factors, the effects of physiological and pathophysiological conditions, and the dynamics of pseudonormalization, all of which affect the evaluation. This article will review the most recent data essential for the proper performance and interpretation of a transthoracic or transesophageal Doppler echocardiographic examination of diastolic function.

Contraindications↗
PubMed1983

Pathological aspects of endomyocardial fibrosis.

The studies leading up to the unitarian concept suggesting that endomyocardial fibrosis, described in the tropics, and Löffler's endocarditis parietalis fibroplastica (Löffler's endomyocardial disease), described in the temperate zone, belong to the same disease spectrum are detailed. Evidence that the eosinophil is involved in the pathogenesis of endomyocardial diseases, irrespective of the geographical origin, is presented. The findings of a similar abnormality in these cells obtained from patients in the tropical and temperate zones are briefly mentioned. In view of the evidence it is proposed that endomyocardial disease, associated with abnormal eosinophils, hitherto classified under the restrictive type of cardiomyopathy, be removed from this classification and reclassified under 'specific heart muscle disease'.

Endocarditis↗
PubMed1995

Feline endomyocarditis and left ventricular endocardial fibrosis.

A retrospective study was conducted of all feline necropsies over a 7-year period. Of a total of 1,472 necropsies, 37 cases of endomyocarditis (EMC) and 25 cases of left ventricular endocardial fibrosis (LVEF) (previously called restrictive or intermediate cardiomyopathy) were identified. There was a subset of four cats with EMC that had histologic features of both diseases. Interstitial pneumonia was seen in 25 of 35 cats (71%) with EMC but in only seven of 25 cats (28%) with LVEF. Thrombi or thromboemboli were seen in 14 of 25 cats (56%) with LVEF but in only six of 37 of cats (16%) with EMC. In both LVEF and EMC, thromboemboli were located in the abdominal aorta, left atrium and ventricle of the heart, femoral artery, cranial mesenteric artery, liver, pulmonary artery, jugular vein, or a meningeal vessel. Each cat had a single thrombus/thromboembolus, except for four cats with LVEF that had more than one. The histologic and clinical findings suggest that EMC and LVEF represent temporally different manifestations of a single disease entity.

Animals↗
PubMed2004

Atrial fibrillation in cats: 50 cases (1979-2002).

OBJECTIVE: To determine signalment, clinical signs, diagnostic findings, treatment, and outcome for cats with atrial fibrillation (AF). DESIGN: Retrospective study. ANIMALS: 50 cats. PROCEDURE: Medical records of cats that met criteria for a diagnosis of AF (ECG consisting of at least 2 leads, clear absence of P waves, supraventricular rhythm, and convincingly irregularly irregular rhythm) and had undergone echocardiography were reviewed. RESULTS: There were 41 males (37 castrated) and 9 females (7 spayed). Forty-one were of mixed breeding; 9 were purebred. Mean +/- SD age was 10.2 +/- 3.7 years. The most common chief complaints were dyspnea, aortic thromboembolism, and lethargy. In 11 cats, AF was an incidental finding. Mean +/- SD ventricular rate was 223 +/- 36 beats/min. The most common echocardiographic abnormalities were restrictive or unclassified cardiomyopathy (n = 19), concentric left ventricular hypertrophy (18), and dilated cardiomyopathy (6). Mean +/- SD left atrial-to-aortic diameter ratio (n = 39) was 2.55 +/- 0.80. The most common thoracic radiographic findings were cardiomegaly, pleural effusion, and pulmonary edema. Median survival time (n = 24) was 165 days (range, 0 to 1,095 days). Eight of 24 cats lived for > or = 1 year after a diagnosis of AF was made. CONCLUSIONS AND CLINICAL RELEVANCE: Results suggest that AF occurs primarily in older adult male cats with structural heart disease severe enough to lead to atrial enlargement. Atrial fibrillation in these cats was most commonly first detected when signs of decompensated cardiac disease were evident, but also was commonly identified as an incidental finding.

Age Factors↗
PubMed1991

Primary cardiac amyloidosis.

Cardiac amyloidosis is an uncommon and often unrecognised cause of cardiac failure. It is an infiltrative disease that may mimic either a restrictive or hypertrophic cardiomyopathy, constrictive pericarditis, coronary artery disease or valvular heart disease. The diagnosis should be suspected in a patient with cardiac failure who has low voltage complexes on the electrocardiogram, in association with increased myocardial mass and echogenicity on the echocardiogram. The definitive diagnosis, however, can only be made by endomyocardial biopsy or biopsy of any involved organ in systemic amyloidosis. Prognosis is poor and treatment ineffective.

Adult↗
PubMed1986

[Radiation-induced cardiopathies].

Pericardial lesions are the most frequent complications of thoracic radiotherapy; they occur in 2% to 30% of the cases depending on the publications. Acute pericarditis, which is the most common form, develops early or late and usually has a favourable course. Chronic pericarditis is divided into chronic pericardial effusion, the incidence of which is underestimated as it produces few or no symptoms, and chronic constrictive pericarditis, itself divided into 2 subgroups of different prognosis depending on the presence (pure fibrous pericarditis) or absence (constrictive sero-fibrous pericarditis) of underlying myocardial lesions. The incidence of myocardial lesions ("myocarditis") varies from 4% to 13% in the literature. They have a minor clinical form characterized by arrhythmias or disorders of conduction and a major form as restrictive or congestive cardiomyopathy with or without cardiac failure. Lesions of the coronary vessels are probably underestimated in view of the results of recent necropsies. Radiation-induced vascular lesions and hyperlipidaemia seem to act synergetically in the genesis of atherosclerosis. Cardiac valve lesions are even less frequent, but here again their incidence seems to be underestimated by conventional diagnostic methods. Echocardiography, radionuclide angiography and exercise tests appear to be useful for the long-term monitoring of patients who had their chest irradiated.

Acute Disease↗
PubMedDate not supplied

Pathology of the cardiomyopathies.

The following article reviews the anatomic and pathologic basis of various cardiomyopathies and illustrates the dilated, hypertrophic, and restrictive types in various echocardiographic planes. Two types of cardiomyopathy are defined: (1) heart muscle disease of unknown cause (idiopathic, primary) and (2) heart muscle disease of known cause or associated with disorders of other organ systems (secondary). Of the cardiomyopathies of unknown cause, the dilated type is the most common. The anatomic diagnosis of hypertrophic cardiomyopathy is based on several morphologic features, the most important of which are asymmetric septal hypertrophy, small left ventricular cavity, and myocardial fiber disarray. Endomyocardial fibrosis with or without peripheral eosinophilia represents the unknown type of restrictive or obliterative form of cardiomyopathy. Selective specific heart muscle diseases (amyloid, iron, myocarditis, toxic, storage disease) are briefly reviewed.

Cardiomyopathies↗
PubMed1987

[Mechanocardiography findings and hypertrophic and dilated cardiomyopathy].

On 71 patients with hypertrophic cardiomyopathy with and without measurable left-ventricular outflow tract gradients, with typical and atypical morphology (ascertained by catherisation of the right and the left heart and angiocardiography as well as selective coronarography) as well as also by catheter investigation including the cardiography of ascertained dilatative cardiomyopathy in 19 patients mechanocardiographic (systolic time analysis from the carotid sphygmogram as well as parameters of the diastole from the apex cardiogram) investigations concerning the heart function and the course were performed. The hypertrophic cardiomyopathy is characterized particularly by a restricted diastolic function, when a good or very good pumping function is present. On the basis of the mechanography the dilatative cardiomyography particularly shows a bad pumping function with also restricted diastolic function. The two forms of cardiomyopathy are clearly to be separated by means of the methods used. Prognostic statements are possible only with reservation. Apexcardiographic parameters are here better suitable.

Adolescent↗
PubMedDate not supplied

Assessment of left atrial appendage flow velocity and its relation to spontaneous echocardiographic contrast in 89 cats with myocardial disease.

The hypotheses of this prospective study were that (1) left atrial appendage (LAA) blood flow velocities can be recorded in cats with myocardial disease by transthoracic Doppler echocardiography, (2) LA enlargement, LA mechanical dysfunction, and left ventricular (LV) diastolic abnormalities are associated with decreased LAA flow velocities, and (3) low LAA flow velocities predict the appearance of spontaneous echocardiographic contrast in cats with cardiomyopathy. Transthoracic 2-dimensional, M-mode, and Doppler echocardiographic studies were performed in 89 cats with hypertrophic, restrictive, dilated, or unclassified cardiomyopathy or with hyperthyroid heart disease. Maximal LAA flow velocity (LAAmax) was decreased (P < .001) in cats with cardiomyopathy (median, 0.28 m/s; range, 0.08-1.35) compared to normal cats. Associated with decreased LAA flow velocities were increased LA size, decreased LA function, increased severity of LV diastolic dysfunction, and the presence of congestive heart failure. Multivariate logistic regression analysis detected an LAAmax <0.20 m/s as the only independent variable to predict LA spontaneous echocardiographic contrast (odds ratio, 30.1; 95% confidence interval [CI], 4.1 222.3; P < .001). Receiver operating characteristic analysis performed to predict spontaneous echocardiographic contrast indicated an area under the curve of 0.88 (95% CI, 0.80-0.95; P < .001) with sensitivities of 100 and 74% and specificities of 69 and 83% for LAAmax <0.25 and <0.20 m/s, respectively. Thus, low LAA flow velocities identified a subgroup of patients at increased risk of spontaneous echocardiographic contrast and possible thromboembolism. These findings may have important clinical implications for anticoagulation therapy and prognostication in cats with cardiomyopathy.

Animals↗